Exciting New Research

(all photos by Jennifer Chase)

I was chosen as a patient fellow for the ALS/MND International Symposium in Boston in early December. I was looking forward to learning a lot and meeting researchers and the other patient fellows.

The day before we were going to fly across the country to Boston, my husband, Stan, was hospitalized with life threatening septic shock. This forced me to cancel my trip to the conference.

But through social media and the ALS/MND International Symposium phone app, I got a message from a woman named Jennifer Chase, who has the C9ORF72 gene mutation like me. Jennifer lost her mom (who had FTD then ALS) and sister to ALS and her sister was one one of the first commercially tested patients confirmed to have the C9ORF72 gene. Jennifer is a brave sister in that she decided to get tested herself. She does have the gene mutation and has been learning all she could since finding out and she has been participating in research studies. She was attending the meeting on her own and she agreed to send me photos and updates. I appreciate her so much because she allowed me to learn through her eyes. What follows is a summary of the updates she sent me.

the registration table

There was a display of ALS/MND patients from all represented countries, including the awesome Osiel Mendoza from the USA.

Jennifer also sent me a photo of the registration swag.

The swag included the Nature Outlook from which I quoted two weeks ago

And now her report on the first day:

The Opening keynote speaker, Dr. Jeffery Rosenfeld from Loma Linda University, spoke about the need for a new paradigm: the need to define and stratify as done in MS, epilepsy, and headache. Even if lines are drawn wrong at first it’s time to start. He also said that biomarkers are what need to be tracked in clinical trials. The patient’s functional rating scale (FRS) doesn’t give results quickly enough.

Then there were awards:

  • Humanitarian award to the guys that started the ice bucket challenge – Anthony Senerchia, Jr. (awarded posthumously), Patrick Quinn, and Pete Frates. Pete’s mom Nancy accepted the award.
  • Forbes Norris Award to Dr. Merit Cudkowicz of Massachusetts General Hospital (and Jennifer’s neurologist) for her passion in researching and caring for patients with ALS and finding the cure.
  • The Paulo Gontijo Research Award to Marta Van Blitterswijk, MD, Ph.D of the Mayo Clinic in Florida for her work attempting to understand the variability among patients with ALS, including age at which it starts, presence of dementia, diesease progression and survival. Most of her patient centered work has focused on the C9ORF72 mutation.

Then Jennifer sent me this photo of ALS TDI researchers. The two on the right are working on C9ORF72 research

She said the rest of the morning was good, although nothing specific to C9 was covered. The Symposium registration was the highest ever – 1281. The scientific sessions were packed. She even saw people on chairs outside the rooms. She said she thought there were slightly more people with ALS there than 2 years ago.

The first afternoon session had a lot about the nuclear pore membrane and C9ORF72. One of the sessions was about SRSF1 (a protein coding gene and nuclear export adaptor) and C9 as a potential way to block the deleterious C9 while allowing the good C9 to work. They are looking at three potential approaches in fruit flies.

She walked through the posters not during the poster session, because of being too tired – I know I would have been exhausted . She noted one that described ALS as the nice person’s disease, and the poster said it looks like some truth to that because the characteristics that make people with ALS nice may be associated with risk factors in lifestyle and occupation.

She went to the clinical trials session on Friday afternoon.

  • Mastinab – normal progressors with less than a 1.1 decline on their FSR showed a slowing of decline at highest dose. There are reports of study irregularities though.
  • Tirasemtiv failed but a related drug is still in trial.
  • Ibudilast – Jennifer said it was confusing. She said they are hoping it slows FSR decline but it needs more study.

Moving on to the Saturday morning session, there was a hopeful presentation on a mechanism to help drugs bypass the blood brain barrier.

She said the biggest deal was Frank Bennett who is Vice President of Research for Ionis, in Carlsbad, CA, which is one of two companies developing the antisense drug. It is already being tested on ALS patients with the first gene defect identified – SOD1 and Bennett said the trial is going well. He also showed a video of kids with Spinal Muscle Atrophy who have been treated and the results were amazing – they were able to meet developmental milestones that untreated patients never could.

Another takeaway was from a presentation from Dr. Michael Benatar of the University of Miami who has been doing pre-familial ALS research – there may be a biomarker sometime that can detect disease before it is obvious clinically which would be a window for treatment for carriers. That would be very exciting.

C9 ALS normally has an average progression rate, but there are subsets – one where there is little progression for years (10 or more years) and another subset where progression is brutally fast. The doctor who was doing extensive genotyping discovered that these brutally fast progressors have a “rare deleterious variant” of the C9 mutation – they all died within a year.

Jennifer also went to Dr. Nick Maragakis’ presentation on excersise. She said basically supervised exercise (stretching, resistance, or cardio) did not hurt the study group but it not help ALS either. But I know from experience that exercise can help quality of life.

Then on to Sunday, the last day. She said there was lots on basic C9 research. Dr. Marka Van Blitterswelk (who won the research award) spoke about her findings on post mortem studies of C9 FTD with and without ALS. It was all good science and increased understanding. This work could not have been completed without the brave C9 patients who donated their brains, and their brave families.

Dr. Nazem Atassi presented on a PET MRI study which is not C9 specific but he thinks it can be used for smaller, faster phase 1 and 2 trials because it has a strong signal.

Dr. Brown giving the closing remarks

The closing session speaker was Dr. Robert Brown, of UMass Memorial Medical Center in Worcester, MA, who has been studying ALS with a special interest in genetics for a long time. He said the C9 antisense trial will be coming up in late 2018 (although I have since heard it may be sooner, and I hope to participate).

I had two comments from family caregivers from my blog requests before the conference.

The first one is from the sister of an ALS patient in California. He was diagnosed in March with familial ALS. After finally getting approved for Radicava he changed his mind so he could apply for the Nurown Phase 3 stem cell trial, and one of the requirements was to not be taking Radicava in the 30 last days. His breathing is also declining quickly and he won’t qualify for the trial if his breathing is below 60 percent Forced Vital Capacity. His sister would like to see changes in trial criteria. Why should a patient have to give up a promising treatment to participate in research? Of course trials are controlled scientific studies but it can lead to very frustrated patients and families.

I too have had frustrations with clinical trials. Many of them exclude patients with feeding tubes. That makes me think that the drug being tested might not be available to patients with feeding tubes.

Cathy Collet, a patient fellow committee member, reported that neurologists are skeptical about Radicava’s efficacy because of the small clinical trial, but glad to have it in their tool bag. I am taking a break from Radicava treatment to sort out unwanted side effects or whatever is causing my sinus problem.

The next comment was from the daughter of an elderly ALS patient in Canada. She is frustrated by the lack of studies on geriatric patients, and the tools to explain feeding tubes and suction to an 88-year-old. She also has observed that weight loss and slurred speech in the elderly is usually attributed to stroke. Cathy Collet said there was no mention of diagnosis in the elderly, and that is near and dear to her heart because her mother was diagnosed in her 70’s. It is an issue that sorely needs to be addressed.

I am sorry that I was not able to attend but I am so appreciative of Jennifer and Cathy for sharing information.

the end of our Hawaii trip and back home to child proof

On Sunday night Jonika and Ollie went to a birthday party.

all dressed up for the birthday party

Stan and Andy and I went to the restaurant at the Kona Country Club. We had a great view of Keauhou Bay and the dive boats with their lights for manta ray diving.

On Monday, our last full day in Hawai’i, we were treated to a whale watching tour on the Body Glove Boat, which is where Jonika and Chris met when they both worked in the boat.

Jonika and Andy, cousins, enjoying the boat
Ollie with dad Chris

We got very close to the whales, and one even bumped the boat! I saw a baby one swimming next to mom. They winter in Hawai’i for mating and giving birth, then make a beeline for Alaska for the summer. We had a naturalist telling us all about them.

there were Spinner Dolphins swimming around too.

The naturalist told us all about the whales’ mating rituals, and we realized we had the results of the Body Glove Boat mating ritual with us. And we love them.

That night we went out for our farewell dinner at Lava Java, a nice ocean front venue with the sound of crashing waves.

Then Tuesday we packed up and Chris and Jonika and Ollie came over to help us clean up the condo and get packed up. With a very short connection in L.A., we knew we had to pack for an extra day, including my food. We put all our meds and toothbrushes in my carryon bag.

When we got to the airport we didn’t have much time to spare. They were lining up the pre boarders. But they soon announced a delay. And then more delay. And we were in the open air airport in the voggy wind. They kept announcing, “no updates, please continue to wait”. We finally made our way to the enclosed restaurant. And then we saw that something had been announced. Stan went to find out. I saw the flight crew leaving as Stan came back and said the flight was canceled. American would put us up at the Hilton Waikiloa with meal vouchers and taxi vouchers. We and all the other passengers had to stand in line for our vouchers and to rebook. We were talking to the people around us and we made a new friend, Anssi from Finland. He had been there for an IT convention. The agent found us a flight the next day on Alaska Airlines which would get us home that night. Our original flight was rescheduled for 4 pm the following day which would get us to Los Angeles after the last flight to Reno.

We rode in a great taxi for the 1/2 hour ride up to Waikiloa. The driver was the owner of a fleet and he said he or another driver would pick us up at 10:30 the next morning.

Our room was on the ground floor right in front of the dolphin pool. Andy called Chris and he said “Sweet!” and said he and Ollie would come early to swim.

We had dinner in a sports bar and Anssi walked by so we invited him to sit with us. We learned a lot about him and Helsinki. He is a sailor and a skier like us. He had been to Reno for gambling and skiing at Squaw Valley. He learned a lot about us too! We will keep in touch.

The next morning Chris and Ollie showed up bright and early. We went to brunch on American Airlines dime.

Then Andy and Chris and Ollie went swimming and Stan and I packed up. On the way out of the hotel there was a man with a parrot.

Our taxi was waiting at 10:30 AM.

And then we flew off the island.

if you look closely, in the distant left you can see snow up on Mauna Kea

Then we finally arrived home and had a few days of sleeping late on Hawai’i time. We also had to do major baby proofing because Jonika and Ollie will be here on January 16, coming to stay with us for a month. Jonika was shaken by Stan’s septic shock as we all were, and we were all reminded that life is short.

Jonika’s best friend, Lyndi, has a son the same age. Jonika said Ollie and Landon meeting will be a dream come true.

they made it! a quick lunch after 2 long flights overnight

I was hoping that my sinuses would clear up by getting out of the vog. But they are still draining. I hope to see an Ear, Nose, and Throat doctor soon.

I also started my fourth round of Radicava and my motion sensors for the precision medicine program arrived. I do have to spend a lot of time managing my illness, leaving little time for pleasure things, like knitting or playing piano and I wonder when I will be able to go back to aqua fitness. I decided to pick up my knitting and the meditative aspect of helps me ignore my sinus problems. I posted a long time ago that ALS is a disease of ch-ch-ch-ch-changes. And life is full of the same.

Even Sue, the T Rex from the Field Museum in Chicago, where Stan and I both saw her as kids, is visiting Reno at our Terry G. Wells Discovery Museum.

The Best Laid Plans (again)

​The best laid schemes o’ Mice an’ Men 
Gang aft agley,

An’ lea’e us nought but grief an’ pain,

  For promis’d joy! 

From Robert Burns To a Mouse 

Source:The Poetry Foundation 

This poem was most famously used by John Steinbeck in the title of his book  Of Mice and Men. The modern version of this quote is The best laid plans of mice and men will often go awry.

Well both Robert Burns’ version and the modern version of that quote fits my Patient Fellows experience for the ALS/MND International Symposium in Boston. It was all planned as a great trip with visits with relatives and dinner with the Patient Fellows who I have gotten to know in conference calls and emails and social media, and also a water workout with the amazing ALS athlete Andrea Peet. The day before we were supposed to fly to Boston, my husband, Stan, got a high fever and ended up in the Emergency Room with severe sepsis. So obviously we had to cancel our trip. Septic shock is life threatening and we got Stan to the hospital just in time, thanks to good friends I was able to text and who came right over and took over.  They called 911 and helped me get Stan’s bipap ready to take with us.

He was in the hospital for a week and it shook me to the core that he almost died. And it was hard on our son getting ready for Junior year final exams.

Stan survived thanks to antibiotics. And “what doesn’t kill you makes you stronger” is usually true. However, it is his medications for his Sarcoidosis that are making him susceptible to infections. Long term prednisone use weakens the skin and his wounds don’t heal without intervention. Unfortunately he will now have to stop taking Humira, which was our best hope for getting him off prednisone, and that is terribly disappointing.

But back to the Patient Fellows experience. Through the ALS/MND International Symposium meeting app on my phone, another woman who has the C9orf72 gene mutation messaged me. When I told her I had to cancel she agreed to send me updates throughout the conference. She loves science and loves to write, so her updates were great and I will be able to share her insights. Also, one of the patient fellow committee members will be sending me notes on some of the sessions I wanted to attend. So I will be able to do a blog about the meeting without having been there.

For the people who contacted me with comments for researchers, I was able to forward them to one of the Patient Fellows group members. So hopefully I will be able to address your concerns.

I was disappointed to not visit Aunt Candy and Uncle Bill, nor my godmother Alice, nor my cousin Len. I was really looking forward to seeing them.

However I also got antibiotics for a sinus infection this week, and I got handicap license plates due to my shortness of breath and the progressive nature of my disease. That will be a big help with my shortness of breath and carrying my portable cough assist and suction.

It was difficult single parenting my 17-year-old while Stan was in the hospital. But wonderful friends brought food for Andy and Erika came and stayed one night and my dad ran errands for me.

I started Round three of Radicava on Friday and will finish this round in Las Vegas on Christmas Eve, where we will spend Christmas with Stan’s dad and step mom.I am still able to care for my houseplants and my jade plant is blooming! That makes me happy.

We had a happy early Christmas celebration with my dad and Anita Friday night and that also made me happy.

We are blessed with great friends and great family. Happy Holidays to all, and especially all people with ALS and their families.

Through the holidays I will plan a blog post every other Monday, so my next post will be New Years Day. Here’s to breakthroughs in ALS in 2018.

I will end with another Robert Burns poem.

Should auld acquaintance be forgot,
And never brought to mind?
Should auld acquaintance be forgot,
And auld lang syne?

For auld lang syne, my dear,
For auld lang syne,
We’ll tak a cup o’ kindness yet,
For auld lang syne.

And surely ye’ll be your pint-stowp,
And surely I’ll be mine!
And we’ll tak a cup o’ kindness yet,
For auld lang syne.

For auld lang syne, my dear,
For auld lang syne,
We’ll tak a cup o’ kindness yet,
For auld lang syne.

We twa hae run about the braes,
And pu’d the gowans fine;
But we’ve wandered mony a weary fit
Sin’ auld lang syne.

For auld lang syne, my dear,
For auld lang syne,
We’ll tak a cup o’ kindness yet,
For auld lang syne.

We twa hae paidled i’ the burn,
Frae morning sun till dine;
But seas between us braid hae roared
Sin’ auld lang syne.

For auld lang syne, my dear,
For auld lang syne,
We’ll tak a cup o’ kindness yet,
For auld lang syne.

And there’s a hand, my trusty fiere,
And gie’s a hand o’ thine!
And we’ll tak a right guid-willie waught
For auld lang syne.

For auld lang syne, my dear,
For auld lang syne,
We’ll tak a cup o’ kindness yet,
For auld lang syne.

Auld Lang Syne a Christmas & New year poem by Robert Burns
Source: https://m.carols.org.uk/auld_lang_syne_burns.htm

Thanksgiving in the U.S. – what I am thankful for

Last Thursday, November 23, was Thanksgiving in the U.S. It is a traditional four day weekend but our local school district added the day before a few years ago, so now it’s a five day weekend. 

We drove to my sister Beth’s in California on Wednesday. She lives south of San Jose, in San Martin. My dad and stepmother, Anita, also drove from Reno. Beth and her husband Jamie have a beautiful home overlooking the CordeValle golf course and a vineyard. Jamie’s sons Phillip and Jack were there too. Because of not quite enough beds, Stan and I stayed at the golf club.

The view from our room.

We had a lovely Thanksgiving dinner at the club.

The beautiful menu – the food was equally beautiful. Jamie’s last name should have a capital B. That is the opposite problem from ours – the small d Macdonalds.

My dad, Phillip, Jamie, Jack, Beth, me, Stan, Andy, and Anita

Although I could not eat the turkey or any of the other wonderful food, I got into the turkey day spirit by drawing a turkey on my feeding tube. Thank you Laura Furumoto for the idea!

We went around the table and each said one thing we are thankful for. Among the comments: indoor plumbing, antibiotics, the first amendment, and the family we were sharing dinner with. Although it is hard to prioritize what I am thankful for, I said I am thankful to still be able self care after nearly two years with ALS. That is not just selfish because it impacts my son and husband greatly. I also seconded the comments about being thankful for everyone at that table. In addition, I am thankful for all of our relatives who we were not with on Thanksgiving.

I am thankful for my friends. Erika and her daughter Maddie came up from Eldorado Hills and spent Friday night and part of Saturday with us. Andy had to sell Christmas trees at his Boy Scout lot at Shoppers Square. Erika bought a tree and I bought a wreath. We will be with Stan’s dad and stepmother for Christmas so we don’t need a tree.

Marvelous Maddie with our wreath
Erika and Maddie with the tree on top of their car to drive over Donner Summit, with Andy and Cooper, who sold her the tree.

Erika even hung the wreath for me.

There are so many friends I am thankful for: the ones from my elementary school, the ones from high school, the ones from college and grad school, and all the friends I have met since I moved to Reno, and also the ones I met online that have become flesh and blood friends.

I am thankful for Radicava and the hope it brings for slower progression of my disease. I am thankful for all the researchers around the world who are working for an end to ALS. I am thankful for all the people who work in ALS clinics to help ALS patients have better quality of life. I am thankful for all the wonderful people with ALS and the wonderful caregivers I have met through ALS fundraisers, Facebook, and our local support group. I am thankful for the people who facilitate our support group.

I am thankful to be a patient fellow for the ALS/MND International Symposium in Boston December 8th through December 10th. I again encourage anyone with questions or comments about anything related to ALS/MND that you want the researchers to hear, please send me your questions and comments. I will be your voice at the conference. Again, you can comment on this blog or on Facebook or on Twitter.

I am also thankful for a fun Twitter interaction. The father of neurology is Jean-Martin Charcot, a brilliant doctor in the late 1800’s in France who first identified and classified ALS, MS and other neurological diseases. Well, Jean-Martin Charcot is on Twitter and he shared my blog! I could not agree more!

 Distraction vs Reality

A couple of my friends who blog about ALS and live in the northern hemisphere (Liz in Canada and Christian in Germany) have written about death from ALS in the last week. The days are getting shorter and the leaves are blowing off the trees. Halloween on October 31st with lots of skeleton decorations and Dia de Los Muertos in Mexico on November 1 (All Souls Day in the Catholic church) are holidays with roots in death even though now they are celebrations. So it makes sense that people think about death this time of year.

I am writing about distraction because it’s a coping method for dealing with a fatal disease. I like to be busy and get frustrated when my allergies cause me to have to be tied to my cough assist and suction machines. I must be allergic to sage brush now and that is a plant that spreads pollen in the wind and it will be flying around until we get a good rainy day. (Which might happen Wednesday if the current forecast is correct). Sagebrush is the state flower of Nevada and the hills around Reno are covered with it. I still have major post nasal drip that gets stuck in the back of my mouth. If I breathe through my mouth I breathe through mucous and can’t get a breath. That is why I am still using the napkin in my mouth. It forces me to breathe through my nose. I have a good nasal spray, Dymista, so I am usually able to keep my nose clear. I have a daily to do list and I try to do at least two things on it each day. So my daily to do list and managing allergies are a big part of distracting me from the realities of how this will end. ALS deaths are usually peaceful. My diaphragm will become less and less effective and then I will have difficulty expelling CO2 and I will sleep more and more. I won’t be gasping for breath. Not a bad way to die. My loved ones can be with me. I feel bad that it will be hard for them but I didn’t write this book and can’t change the ending.

I was disappointed to not be able to go to aqua fitness on Saturday November 11, the first day I could swim after my port surgery on October 10. My allergies were too bad. I have tried nearly every antihistamine on the market. Nothing keeps my throat clear long enough. The trick is to find something without major side effects: One that won’t dry my eyes too severely and one that won’t make me too drowsy. I am now trying Zyrtec and a new formulation of it called XYZAL.  It could take up to a week to be effective but my first 10 ml. dose was effective for about 4 hours.

It is ok to be sad about leaving this wonderful world, and we have to grieve at times. But I think it’s ok to carry on with life and do the things that make you happy while you can. I play piano and I knit and I do aqua fitness and spend time with family and friends and I want to do those things until I can’t anymore. 

But I was having my own pity party when I couldn’t swim on the first possible day back. And then I was inspired by a 30-year-old friend with ALS. Early  Sunday morning I read a blog post from Suuny Braus Erasmus who wrote about Franklin D. Roosevelt, who didn’t let paralysis from polio stop him from becoming the 32nd  President of the United States – which had been his goal before polio. Reading that gave me the kick in the ass I needed and I went to aqua fitness on Sunday morning at 8:45. For the first time I kept my napkin in my mouth the whole class and had to focus on nose breathing. It was not comfortable but I got the workout in and that feels good.

I am so inspired by so many younger people with ALS:

  • Sunny Brous Erasmus, diagnosed at age 28, met her husband at 29 and got married at 30 and blogs at sunnystrong.wordpress.com. She continually inspires me. 
  • Sarah Coglianese, diagnosed with ALS at age 33, who just won the Tradition of Excellence Alumni Award at Oak Park River Forest High School. She gave an impressive acceptance speech about empathy and how it could help many of the problems in this world. She has raised very large amounts of money for ALS research with her #whatwouldyougive campaign (which inspires empathy) and is a gifted writer who inspires ALS patients and others with her blog about ALS speed4sarah.com. Despite me being 18 years her senior, she is my mentor and friend. I went to OPRFHS with her aunt.
  • Corey Reich, who was diagnosed in college at age 21 and has raised lots and lots of money for research in his ten years with ALS and he is still a tennis coach at his high school.
  • Andrea Lytle Peet, who was diagnosed at age 34. She was a triathlete and she continues to compete in marathon races on her recumbent bike to raise money for ALS research and blogs at teamdrea.com
  • Osiel Mendoza, who was diagnosed in college at age 21 last year. He married his girlfriend a couple months ago – they have been a couple since 8th grade. He has already raised a lot of money for ALS research. He gave an inspiring speech at ALS TDI’s fundraiser in Boston a few weeks ago.
  • Eryn Criswell Blythe who was diagnosed at age 36 and has two young children. Her husband is her caregiver. She was pushed in her wheelchair in the L.A. Marathon last summer to raise money for research.
  • And Liz https://lizfeltham.wordpress.com and Cristian https://workandloveblog.wordpress.com are both younger than me I believe, and they both inspire me regularly.

These young people are taking what life has dealt them and are making the world a better place and moving forward with optimism. That is certainly enough to stop my 57 year old pity party!

The only picture this week is from Andy’s Cross Country banquet on Wednesday last week. He got a Varsity letter and he got a coaches award for his hard work and improvement. 

I am proud of him for working hard on running. Now he has to figure out how to keep his grades up while participating in a sport that takes so much time (practice 6 days a week). He wants to do track next semester but he will only be able to if he can bring his grades up this semester (family rule). He did it last spring to be able to compete in cross country so I think he can do it again if he can focus on school. Keeping him on track for track is a distraction too. Maybe all these young amazing people with ALS can inspire him too.
I don’t know when my ALS journey will end and I should be more hopeful since I am on the new drug Radicava, but the shorter days and allergy woes were bringing me down. At least we now know that I am probably not allergic to the Radicava since my symptoms have continued on the off cycle. And all I have to do is think of these young people with ALS and my personal pity parties will be over.

 

Halloween and the end of first round of Radicava 

As promised, I reprised my Beauty that Killed the Beast costume for Halloween, and this time got pictures.

Photos by Andy Macdonald

I had to make a little hole in the dress for my feeding tube so I can eat while wearing it. But since King Kong made such a mess of my dress anyway, the little hole was not noticable.

Our street gets filled with cars bringing kids from other neighborhoods. When the doorbell rings every few minutes there are about ten people each time on the porch. After running out of candy very early one year, we decided to never be home again. Luckily, our good friends Chuck and Alice have an annual Halloween party. Stan reprised his Parrot Head costume but Andy changed his up.

Andy got to trick or treat with the younger kids and he filled up a pillow case.

It was a great party with a little boy who dumped his candy on the floor and his dad went through it with him (classic!)

And Dad wanted to steal the candy (also classic!)

Calvin the little pirate was happy with his treasure.

Calvin’s sister Alana with her great grandparents
Doesn’t Alana look comfy with her great grandpa?

Alice had invited two other friends from aqua fitness, Marilyn and Mary. I was able to give them my update and tell them I will be back November 11.

Marilyn from aqua with Rita, Chuck and Alice’s daughter 

I miss my aqua fitness classes so much. They really do cover the range of motion. I had to take a month off after my port surgery which was on October 10. So on Saturday November 11, I will go back. I do my range of motion exercises home but it is not the same as an hour class in the water. And the first time I did the exercises at home was Nov. 2. I also have my ALS TDI Precision Medicine Program motion sensors that I wear for a week each month and have prescribed movements to do every other day and I started wearing them on Nov. 2. I am feeling it in my muscles but I know it is normal to feel sore when starting an exercise program. It is also scary because cramps and muscle soreness can be a sign of ALS progression. But I have to be ready for aqua fitness on October 11. Taking all this time off of exercise was not good for me. But I had plenty to keep me busy with the extra time.

The Patient Fellows and Committee for the ALS/MND International Symposium in Boston had a conference call where we each shared our interests in sessions we hope to attend. There is nice diversity among us so we won’t all be reporting on the same things. It will be tricky balancing attending all the sessions I am interested in with my energy level. 

Our dryer broke and I was able to call to arrange a service visit. I was home alone when the service guy came and I was able to deal with that using my Boogie Board. That hardly seems like a blog worthy event, but anytime I can handle things like this on my own seems like a victory now. 

We also went to a fun fundraiser for Reno High School. It included raffle, silent auction, buy a balloon for a guaranteed prize, and a live auction as well as sit down dinner. We had a fun table with friends from my work, other good friends, and new friends.

With Rene and Janet who I worked with. Photo by Stan Macdonald
Todd and Judy, my mala bracelet friend. Photo by Stan Macdonald
Justin and Suzi Champagne, new friends. Photo by Judy Harrison 
Photo by Judy Harrison

Also at our table were my friend Melissa, who was the photographer for the event, and her husband Charlie, who we met for the first time. The photographer is never in the photos. Sorry Melissa. I wish I had gotten one of you and Charlie.

As part of the live auction they auctioned off about 10 cakes from the Atlantis bakery. If your table bought a cake, that was your dessert. If not, you got cookies. Stan loves chocolate cake so he won the bid for our table.

Paying the winning bid for the cake. Photo by Judy Harrison 
Photo by Judy Harrison 

It was a fun evening supporting our son’s school. We also scored a silent auction item.

The Fly Away “basket” put together by Reno High staff, includes $200 in Southwest Airlines gift cards and all kinds of travel goodies in a nice travel bag. Photo by Sabine Beach

On November 2, Judy and I were going to go see the movie I’ll Push You about two friends who did the Camino de Santiago, and one was in a wheelchair so his best friend pushed him all the way. This was a fundraiser for MDA shown one night only all over the country. When we tried to get tickets it was sold out. But Judy decided to go anyway to see if she could get in. With my allergy symptoms, I did not want to go without a sure ticket. Stan’s cousin Lisa called and said she was in town so we met her at Great Basin for dinner. At 7 pm, Judy texted and said she got two free tickets and could I buzz down to the theater? But because we were out to dinner I could not hop in the car and go. I do hope to see it someday. Judy said the words love, connection, and hope were used many times in the movie 💜. She also said that the guy in the wheelchair was uncomfortable being cared for by others until he realized that helping was making his caregivers happy. That is a good thing for ALS patients to realize. My friend Sunny Brous (another blogger with ALS) shared this quote:

One of the secrets of life is that all that is really worth the doing is what we do for others. – Lewis Carroll

Last week I also completed Round 1 of my Radicava infusions.

Infusion #14 of Round 1

Now I get 14 days off. In the rest of the cycles I only have to do 10 of 14 days. So I will be able to do 5 on, 2 off, and 5 on then have 16 days off.

The weather forecast is showing the next three nights should have temperatures below freezing. I hope that this will be the end of my allergy season.

And finally, my online friend whose tagline was “I have ALS, ALS doesn’t have me,” passed away after over 10 years with ALS. Rest in peace Andre Williams, Sr. Your positivity and kindness will be missed.

Fun at Corey’s Crusade and Radicava Round 1, Days 1 through 10

On October 21 we went to Clairmont Country Club in Piedmont, California for the annual Corey’s Crusade fundraiser for ALS TDI. Corey was diagnosed in college. This was his 10th annual party (He is now 31). Every year the party has had a different theme. For this party we could choose a theme for a costume from any of the previous 9 themes.

Margaritaville, Safari, the Beatles, Masquerade, Midnight in Paris, Western, Super Hero, Havana Nights, and Purple Rain.

Stan was a Parrot Head for the Margaritaville theme and Andy chose Purple Rain. I was a superhero of sorts, the Beauty who Killed the Beast.

Andy added round purple sunglasses and purple beads that they had at the party. You will have to wait for Halloween for a good photo of my costume. 
A selfie we took on the way in
With Jen from ALS TDI. We love you Jen! You can see the giant gorilla hand around me. But I promise a better photo on Halloween.

It was a great party where we saw friends and met many nice people. And I am sure they raised a lot of money. We stayed in Walnut Creek because we had to be back in Reno for the 1 pm show Beautiful – Carole King the Musical. I did my Radicava infusion and ate my feeding tube breakfast while Stan and Andy went down to the hotel breakfast. Then we packed up quick and hit the road. We made it by 12:55 pm. This was an 85th birthday present for my dad. It was nice to share such a wonderful show with my dad and Anita and Stan and Andy. Even Andy liked the show.

I have now completed over a week of Radicava. The first few nights I felt that I was sleeping better. I have been remembering dreams again. But by the 4th and 5th day I was having runny nose and lots of thin post nasal drip. There was so much I thought it must be an allergic reaction. We notified Searchlight of a possible reaction. But the next day Stan talked to two different pharmacists from Option Care. They said that it is possible to have an initial reaction to port medicine and it would go away with time. They also said see how I feel on the off weeks. So I loaded up on Benadryl and Sudafed, and I am still hoping for a freeze. I am watching those low overnight forecasts. Come on cold weather!

I can’t swim until a month after my port surgery, which will  be November 11. I can’t wait to get back in the pool. I feel better when I am doing all those range of motion exercises. I will have to start doing them at home for the next two weeks. For the next round of Radicava we have a little more flexibility. It will be ten of 14 days so we can do 5 days on then take the needle out and I can have two days to swim and shower without plastic wrap, and then do the second 5 days. We looked at the November calendar and maximized the days I could swim. I also will explore ways to cover the needle so I can do aqua fitness and stay in shallow water.

My friend Erika came up from Eldorado Hills, California for a quick visit. She learned how to do my infusion. I am able to do the infusion myself now but someday I may need help.

I am so blessed to have wonderful friends and Erika is the best of the best!

A Step Back in Time, then a Giant Leap Forward

On a beautiful Saturday in October, we did something we have wanted to do for a long time. We rode the V&T Railway roundtrip from Carson City to Virginia City with friends.

There were plenty of characters dressed up to take us back over 150 years.

It was fun to look for wild horses and old mining facilities and it was also interesting to imagine traveling across the country by steam train.

A dog’s tail and leash between the seat and the back of the seat.

Andy met us in Virginia City because he had to go to cross country practice. We got him a one way ticket for the ride back.

Our gang with St. Mary’s in the Mountains Church in the background: Rita, Maddie, Paul, Alice, Andy, Stan, Chuck, and David
The steam engine chugging away after dropping us off. Photo credit Andy Macdonald
4th Ward School. Photo credit Andy Macdonald

We hiked uphill to lunch at Cafe del Rio. There was a zombie group scavenger hunt going on so it was nice to be on the south side of town. Most of the competitors were in costume but not many looked like zombies. There were a lot of people running around though. After lunch we toured the Chollar Mine. The guide had interesting facts about the mining industry in Virginia City since the 1860’s.

Paul and Maddie in front of the mine entrance. Photo credit Andy Macdonald

After the mine tour, Stan and I had had enough walking and I had enough pollen, so we walked back and sat in the train car with all the other old tired folks.😕 The rest of the group hiked back up to C Street for ice cream. 

Andy enjoyed the train ride back down to Carson City. When we got back to Carson we all parted ways and Andy rode with us to drive back up to Virginia City to get his truck. Even with my allergies it was a really fun day.

Now we will stop in present day for a moment. It was time for my three month feeding tube change and my sweet and funny husband changed it for me.

Now the giant leap into the future: I finally started the first new drug for ALS in 20 years. I call this a giant leap into the future because there are so many promising new drugs in trial or close to trial, so future ALS patients should have treatment options.

Radicava in my hands finally! Photo credit Alice Hilsabeck

We got approved to start home infusions with only one training session because Stan was an EMT. And our friend Alice is a retired nurse and she is willing to be the backup.

Five days of Radicava that would be coming home with us.
I am so lucky to have my wonderful husband and friend willing to help with this.

Our Option Care nurse, Pamela, was an excellent trainer. She explained everything and instructed us in the need for sterility since the drug is going into my Vena Cava through my port. She placed the infusion needle in my port and I was ready to go. Stan learned how to do the infusion.

This and all remaining photos credit Alice Hilsabeck

I am so happy to finally be on this drug. The reported 30 % reduction in progression of ALS is huge. The only other drug for ALS available before this only extends life for a few months. 

For all United States ALS patients still waiting to start, I recommend that you be your own advocate. Call your nurse educator. Call Searchlight Patient Support and ask for your patient ID number and your case number. Then ask to talk to your case manager for the status of your approval. You also may have to call your insurance company, your neurologist’s office, and the infusion company. Make sure that you tell everyone that every day makes a difference in this disease. The local infusion company wanted me to wait until next Wednesday to start three required days of training. But we asked if there was any way to start sooner, for instance, could I come in to their facility? And the answer  was, “Yes, tomorrow.” Woo hoo! The pharmacist did call Stan to talk with him and verify his training. 

Love of friends and family, connection with friends, family, and medical professionals, and hope that this drug will allow me to see my son become a man and allow me to be around for the next ALS drug that comes along –  potentially halting or reversing the disease. Let’s hope.

LOVE, CONNECTION, HOPE!

Every picture tells a story, don’t it?

If you are on Facebook with me then you already saw this. I was challenged to post 7 Black and White photos of my life on 7 consecutive days with no people and no explanation. Here they are:

This week I had my port installed on Tuesday, to be ready for Radicava infusions as soon as my infusion supplier sets it up which hopefully will be this week. My surgery was scheduled for 4:40 pm. They told me I could eat until 6 am, so I got up early and did that. Around 8:45 I got a call asking if I could be there at 9:30 for 11:30 surgery. So I quickly showered and got there at 9:30.

I waited in a children’s room with this kid hanging out with me all day

When the nurses heard I ate at 6, they had to go talk to the anesthesiologist. They decided to do another surgery before mine and said they would try to do mine around 1 pm. So we waited. 1 pm came and went. Stan went to get lunch. They told me my surgery was back on the schedule for 4:40 pm. I asked if I could have more allergy medicine and the answer was no. I actually cried at this point – I was afraid of aspirating on post nasal drip going into sugery. If I had known I would be there all day I would have brought my meds. But they finally said I could take my own Allegra. Stan was having lunch over by the CVS pharmacy and the nurse called him and asked him to pick up some Allegra. He came back and I took it with just a little water. And still we waited. When 4:40 came and went I felt like punching out a few more teeth on that kid hanging with me, although Stan pointed out that it wasn’t his fault, so I controlled that impulse. Ultimately my surgery started after 5 pm. The surgeon apologized for the mix up and all went well.

Post surgery with lots of permanent marker marks and with a port under the bump on my right side.

Then on Wednesday, we found out that my infusions are being held up by the need for a signature of the contracts manager for the infusion company, who was gone all week. I complained that it is 2017 and anyone can sign anything anywhere anytime. But again, we waited through a weekend to make more progress. Radicava Ridiculousness!

We have received great support from MT Pharma America, the drug company. The long drawn out waiting period has been hard, but hope in an infusion bag is closer than ever.

What sort of beast is man?

You belong among the wildflowers

You belong in a boat out at sea

Tom Petty – Wildflowers

Perhaps a better song to quote is U2’s Sunday, Bloody Sunday. The tragedy in Las Vegas was so unthinkable and horrific. We watched the first episode of Ken Burns’ The Viet Nam War this week. A veteran was quoted saying that there is a reason we are the dominant species on this planet and that people say the military turns boys into men, but no, he said, it is just a finishing school. That was a chilling comment to hear after Sunday. In another movie we watched recently there were mountain lions (scary) but the humans in the movie were much scarier. Our whole state was impacted by the tragedy Sunday. It is sad that our country can’t do more for mentally ill people and can’t come to consensus on gun safety. #BanBumpStocks 

Northern Nevada supports you Las Vegas. The love that poured out among those fleeing as well as the first responders and hospital staffers, as well as the response from the community at blood donation sites in both Las Vegas and Reno, shows the side of humanity I would rather focus on. #VegasStrong #NevadaStrong. I wish that everyone impacted somehow finds peace and recovery soon. I know it will be a long time coming. It affects every security guard in casinos in Las Vegas and in Reno, and many, many other walks of life too and every big city outdoor music festival, as well as the friends and family of the victims. And every victim belongs among the wildflowers or on a boat out to sea, instead of being killed by a human beast.

We also lost a rock and roll icon, Tom Petty, a week ago. I like his music and Stan and I saw him live in Reno many years ago. I chose those two lines from his Wildflowers song not only for the victims but also because I love to be outside among the wildflowers, but my seasonal allergies are really bad this year. I can’t cough or blow my nose or swallow well so I am beholden to my cough assist and suction machine. If I could go on a boat out to sea I could get away from the pollen.

I have had a lot of thick saliva. From the British MND Association website I got a PDF about dealing with saliva in ALS/MND.

I have been taking Pineapple and Papaya Enzymes to help with breaking up the thick saliva. The publication said pineapple juice could be swabbed in the mouth to help with the same thing. We had some pineapple juice, so I swabbed some in my mouth. I got a very quick reduction in thick saliva. But it only lasted about 5 minutes. So then I swabbed some more. And then some more. And soon I had swollen lips and felt as if I OD’d on antihistamines. This could be an allergic reaction. It turns out pineapple is a strong antihistamine. Even if it is not a pineapple allergy, I do not want to OD on antihistamine. I had been trying various combinations of anantihistamines and decongestants and expectorants, to find allergy relief and trying to keep track in my head. But there are way too many variables to keep track of. Plus I really want to find out which med is making my eyes uncomfortable. I messaged my doctor at UCSF to get her input. She agreed that I can methodically try discontinuing one med at a time, and stay off any discontinued for at least a week. I decided to start with Neudexta. I can stay off it and see if I am having psuedobulbar affect (inappropriate laughing and crying). If I am not having that symptom, then I can discontinue it completely. Four days off so far and nothing inappropriate. I am also documenting when I need my eye drops. Yes, I still need them, and yes I am still having allergy symptoms (sneezing – works better than cough assist! I appreciate that I can still sneeze), itchy eyes, runny nose. This could all still turn out to be my seasonal allergies. I am still hoping for a deep freeze soon!

I became empowered this week and decided to make my own phone calls. Stan does not have patience to wait for customer service to pick up and he gets frustrated with being passed on to another hold and phone tag is also frustrating to him. So I sat down with my Speech Assistant on my phone with my wireless keyboard and used our house phone to call our insurance company. I had pre-typed “My name is Margaret Macdonald. Please be patient while I type.” I waited on hold a long time and then I got the screener. I explained that I was calling because the accumulator that my providers see says I have not met my maximums but I have. So my doctors make me pay and then we ultimately get refund checks. Then I was connected with someone who could help. She said she could see the problem and she would send it their IT department. Then she said, “is there anything else I can help you with?” And I said, “yes. I am waiting for approval for a new drug and with my disease every day counts.” She said it is all approved and she would call Option Care, the infusion supplier, for me. Success!

Next I called Searchlight, the patient support group for Radicava – the new drug. Again after a long hold and with a pre-typed opening statement I talked with a customer service representative asking for my Patient ID Number and Case Number, and my status. I was transferred to my case manager. She had my benefit summary and she had already enrolled me in the copay support program. I should receive the benefit summary in the mail on Tuesday. Again success! So now I can make my own phone calls, and my husband will be less frustrated.

My allergy struggles and all my ALS struggles are so very minor compared to the hell created by the human beast in Las Vegas. Now if only we could turn the clock back to before that Sunday, bloody Sunday, night…..